Search for a rare disease

Other search option(s)

If you have selected the “Other” category, please specify which type of user you are: *

Email address: *

Topic of your comment *

Epidemiology data

Summary and related texts

Related genes

Clinical signs

Nomenclature and/or coding

Your message *

(3000 characters remaining)

Attention

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Check this box if you wish to receive a copy of your message

Please reproduce the text below: *

Juvenile idiopathic arthritis

Disease definition

Juvenile idiopathic arthritis (JIA) is the term used to describe a group of inflammatory articular disorders of unknown cause that begin before the age of 16 and last over 6 weeks. The term juvenile idiopathic arthritis was chosen to signify the absence of any known mechanism underlying the disorder and to highlight the necessity of excluding other types of arthritis occurring in well defined diseases (in particular arthritis occurring in association with infectious, inflammatory and haematooncologic diseases).

ORPHA:92

Synonym(s):

Juvenile chronic arthritis

Juvenile rheumatoid arthritis

Prevalence: Unknown

Inheritance: -

Age of onset: Childhood

ICD-10: M08.0 M08.1 M08.2 M08.3 M08.4 M08.8 M08.9

OMIM: -

UMLS: C0553662 C1444838 C3495559

MeSH: D001171

GARD: -

MedDRA: 10059177

Summary

Epidemiology

Incidence is estimated at between 1 in 100,000 and 2 in 100,000.

Clinical description

Precise definitions of the diagnostic criteria for the different types of JIA were established in 2001 at the last international meeting in Edmonton. Six disorders have been defined: systemic-onset juvenile idiopathic arthritis (formerly referred to as Still's disease), oligoarticular arthritis, rheumatoid factor-positive polyarthritis, rheumatoid factor-negative polyarthritis, enthesitis-related arthritis (spondylarthropathies), and the juvenile form of psoriatic arthritis (see these terms). A seventh category has been defined comprising unclassified types of arthritis (types that do not correspond to any of the defined disease or that correspond to more than one of the disease definitions).

Diagnostic methods

The criteria that define the diseases are mainly clinical, but genetic studies (in particular linkage with HLA antigens) confirm that these are different disorders and not different clinical forms of a single disease.

Management and treatment

Treatment of JIA is best managed in specialised centres where rheumatologists and paediatricians work in collaboration with physiotherapists, paediatric orthopaedists and psychologists to ensure global management of the medical aspects of the disease and its consequences on school and family life. Since 2000, biotherapeutic approaches targeting inflammatory cytokines, such as the tumour necrosis factor (TNF)-alpha inhibitor Etanercept, have revolutionised the management and prognosis of severe forms of the disease. Methotrexate remains the second-line treatment of choice. However, results with methotrexate and anti-TNF-alpha agents in patients with systemic forms of the disease have been disappointing. For these patients, several other treatments are available and/or under investigation such as thalidomide, an interleukin-1 receptor antagonist (anakinra), and the anti-interleukin-6 receptor (IL-6R) monoclonal antibody (MRA). Given this range of treatment options, it is very important to provide early specialised care so that treatment can be adapted to each child. In addition, there is also a risk of uveitis, which may evolve without symptoms or eye redness but may have severe consequences in the absence of early treatment. Thus, regular ophthalmologic monitoring, including slit-lamp examination of the eyes, should be performed every three months for young children with oligoarticular arthritis or rheumatoid factor-negative polyarthritis.

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

With the support of

Our Website does not host any form of advertising
Our partnerships do not influence our editorial policy